We feel that a CD4+ CD26- percentage value higher than 30% of peripheral blood lymphocytes could correctly identify the presence of peripheral blood involvement in SS and MF patients.

Two low-/high-risk groups have been singled out on the basis of the risk index. Patients with no or one adverse prognostic feature(s) (risk index < or = 1; n = 31) share a slow disease course and a relatively favorable prognosis (five-year survival: 58%); on the other hand, patients with 2 or 3 adverse prognostic feature (risk index > 1; n = 20) are characterized by an aggressive disease course not modifiable by traditional therapies (five-year survival: 5%).

Serial immunologic tests (active E‐rosettes = T‐Et; total E‐rosettes = T‐Et; total lymphocytes and null cells) were performed every 3 months for 5 years on 113 melanoma patients. A significant reduction in absolute T‐Ea, T‐Et, null cells, and total lymphocytes was noted in the patients who died, by comparison with those who are still alive. The latter presented a significant reduction in absolute T‐Et only, plus a significant increase in null cells when compared with normals. The 38 patients without metastases, at the end of the study, presented a reduction in T‐Et and an increase in null cells compared with the normals, while the 75 patients with metastases presented a reduction in T‐Et, null cells and total lymphocytes when compared with the patients without metastases and a reduction in T‐Ea, T‐Et, and total lymphocytes when compared with the normals. Null cells show a linear decrease in patients who died and a linear increase in those who survived. A total of 80.2% of patients with a fall in T‐Et displayed metastases usually within 2 to 10 months (mean, 6.8). Patients with normal T‐Ea, T‐Et, and total lymphocyte values showed a significant prolonged survival when compared to those with lower values. In addition, survival seemed to be always a function of immunologic test values, irrespective of the tumor site.

T and B lymphocyte populations were evaluated in forty-one patients with multiple recurring warts. Active rosettes (T-Ea) and total T lymphocytes (T-Et) were significantly decreased. The in vitro effect of a calf thymus extract (TP-I) on peripheral blood lymphocytes was evaluated in thirty-three patients. A statistically significant increase in E-rosette forming cells was observed in twenty-three patients (69%). Serum blocking factors were not found in the patients with a positive response to TP-I, which suggests the possibility of a thymic hormone deficiency. In the case of non-responders to TP-I, on the other hand, an anti-lymphocyte antibody capable of directly blocking the hormone receptors on the undifferentiated lymphocytes may be postulated.

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BackgroundSystemic juvenile idiopathic arthritis (sJIA) is a rare autoinflammatory disease of unknown etiology. Several uncontrolled studies showed that early treatment with anakinra is associated with a better outcome, according to the “window of opportunity” hypothesis. However, very limited scientific evidence is available on withdrawal strategy. So far, anakinra withdrawal modalities are heterogeneous among the different rheumatology centres.ObjectivesThe aim of this study was to identify predictive factors of disease flare, as elements suggestive of persistent course of the disease, in patients with sJIA after anakinra withdrawal. We also described our withdrawal strategy in sJIA patients who reached clinical inactive disease (CID) off glucocorticoids (GCs).MethodsWe retrospectively analysed data of 39 sJIA patients followed in our centre who stopped anakinra after reaching CID off GCs for at least 6 months before the withdrawal. Eight patients withdrew anakinra abruptly while 31 patients did it after tapering throughout one or two steps (alternate-day and/or once every three days regimens) of anakinra administration. All patients underwent a follow up of 24 months after withdrawal. They were subsequently divided into two groups according to the presence or the absence of disease flare during the follow up. Demographic, clinical and laboratory data of the patients were evaluated in univariate and multivariate analysis as predictors of flare.ResultsDuring the follow up, 10/39 patients (25.6%) flared after a median time from anakinra withdrawal of 7.9 months. In univariate analysis the variable most strongly related with the absence of flare was the disease duration from the onset to starting of anakinra (p = 0.0046), with an optimal cut-off of 3 months. Patients who started anakinra ≥ 3 months after disease onset had an 8-fold higher risk of flare after anakinra discontinuation (CI 95 2.0-32.4; p = 0.0003) (Figure 1). Furthermore, we observed that a higher dose of anakinra (≥2 mg/kg/day) was associated with a lower risk of flare (p = 0.065). We also demonstrated that patients who tapered therapy before withdrawal were significantly associated with a lower percentage of flare compared to the ones who withdrew anakinra abruptly, with a 4-fold lower risk of flare (CI 95 1.47-10.1; p = 0.0164). Lastly, we constructed a model analysing at the same time disease duration, anakinra dose at baseline and anakinra tapering. In this model, disease duration ≥ 3 months at baseline resulted to be the only variable significantly associated with flare after anakinra withdrawal with an Odds Ratio of 15.16 (CI 95 1.7-131.9; p = 0.014).ConclusionOur results strengthen the evidence on IL-1 inhibition in sJIA treatment and provide new ones supporting the “window of opportunity” hypothesis. Early anakinra treatment may predict a good short-term outcome and also potentially prevent the development of a persistent disease course. These observations have a strong clinical relevance, an early diagnosis and a targeted treatment could control the disease and could also modify its natural history.References[1]Masters SL, Simon A, Aksentijevich I, Kastner DL. Horror autoinflammaticus: the molecular pathophysiology of autoinflammatory disease. Annu Rev Immunol 2009; 27:621-68.[2]Nigrovic PA. Review: is there a window of opportunity for treatment of systemic juvenile idiopathic arthritis? Arthritis Rheumatol 2014; 66:1405-13.[3]Pardeo M, Rossi MN, Pires Marafon D, Sacco E, Bracaglia C, Passarelli C, et al. Early Treatment and IL1RN Single-Nucleotide Polymorphisms Affect Response to Anakinra in Systemic Juvenile Idiopathic Arthritis. Arthritis Rheumatol 2021; 73:1053-1061.Figure 1.Relationship between disease duration from disease onset to starting of anakinra and rate of flare within the 24 months follow up after anakinra withdrawal.Acknowledgements:NIL.Disclosure of InterestsGermana Nardini: None declared, Claudia Bracaglia Speakers bureau: SOBI, Consultant of: SOBI, Novartis, Denise Pires Marafon: None declared, Emanuela Sacco: None declared, Arianna De Matteis: None declared, Ivan Caiello: None declared, Giusi Prencipe: None declared, Fabrizio De Benedetti Consultant of: SOBI, Abbvie, Novimmune, Novartis, Roche, Pfizer, Manuela Pardeo Consultant of: SOBI.